Specialized Care for Primary Immune Deficiency

Apr 25, 2017

In recognition of World PI Week, learn how Accredo’s team of nurses and pharmacists help patients with primary immunodeficiency diseases manage safe and effective treatment to fit within their lifestyle.

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Immune Deficiency

The immune system is the first line of defense in protecting the body from infections and diseases. When part of the immune system is missing or functions improperly, Accredo’s dedicated team of nurses and pharmacists are the patient’s next line of defense.

Primary immunodeficiency diseases (PI) consist of more than 200 rare, chronic disorders affecting approximately 500,000 people in the U.S. These disorders are caused by hereditary or genetic defects. While some disorders are present at birth or in early childhood, disorders can affect anyone, regardless of age or gender. While patients’ PI diseases may differ, they all share one common feature: a deficiency in the functions of the body’s normal immune system, which increases vulnerability to infection.

Patients’ infections can affect any part of their body. Some of the most common reoccurring infections are skin, sinus, upper respiratory, and ear infection. These infections can lead to more severe infections that affect the lungs, brain and other major organs as well as other serious health concerns. This increased vulnerability to infection leaves the patient to have recurrent infections, infections that won’t clear up with treatment, or infections that are unusually severe.

Diagnosing PI can be a lengthy endeavor. Doctors collect information through laboratory testing for deficiencies in the immune system along with reviewing medical history, family history and a physical examination. By looking at the patient’s full picture, a collaborative decision can be made to develop the best strategy of treatment.

According to the Immune Deficiency Foundation, a patient may present infections that are:

Severe: requiring hospitalization or intravenous antibiotics

Persistent: won’t completely clear up or clears very slowly

Unusual: caused by an uncommon organism

Recurrent

Runs in the family

Treating PI

Immunoglobulin (Ig) therapy is one of the few treatment options for patients with PI.

Immunotherapy uses immunoglobulins or antibodies from blood plasma collected from live donors. Individuals can benefit from immunotherapy replacement if they are unable to produce adequate amounts of Ig or antibodies.

How We Help

Patients with PI have a life long journey ahead. However, Accredo clinicians help patients manage their treatment to fit their lifestyle. Here are two recent examples:

A busy college student with a PI disease was unable to keep the all-day infusion appointments at his local infusion center. Through the support of a specialist pharmacist and Accredo’s nursing staff, we transitioned this patient to a home infusion program. He improved adherence to treatment, had no infections and zero missed doses.

Another individual had been taking a vacation day every month for 20 years to receive infusions at an outpatient facility. After Accredo made an outreach, he now handles his infusion independently through weekly 1.5 hour infusions on Saturdays while napping.